A Study of Home Therapy in Hemophilia Patients / 임상소아혈액종양

BACKGROUND: Home therapy has merits, enabling rapid treatment thereby reducing complications, but difficulty with venous access is a barrier to treatment. There is a paucity of data on this. This study investigated the current status of home therapy for patients with hemophilia, and examined problems and potential solutions.METHODS: Patients and their families who were performing home therapy and who attended the camp of the Korea Hemophilia Association in 2014 were asked to complete questionnaires. The questionnaires pertained to complications of infusion and whether (or not) factor infusion was properly performed. Responses were scored on a scale from 1-5, with a high frequency of complications and adequate performance of infusion being allocated relatively higher scores.RESULTS: The mean score of complications arising from infusion was 1.56±0.46. This was relatively low and was not correlated with the factor infusion training method. The performance of home therapy obtained a relatively high score: 4.46±0.56. The performance score was significantly higher for patients who had practiced infusion with medical personnel, an injection simulator or a video clip.CONCLUSION: Although most patients properly performed home therapy, further improvement is needed in training of infusion and keeping records of bleeds. It is essential to establish guidelines on home therapy, develop a standardized patient and family training program, and reinforce the role of hemophilia treatment centers in educating patients and their families.

A Study of Home Therapy in Hemophilia Patients / 임상소아혈액종양

BACKGROUND: Home therapy has merits, enabling rapid treatment thereby reducing complications, but difficulty with venous access is a barrier to treatment. There is a paucity of data on this. This study investigated the current status of home therapy for patients with hemophilia, and examined problems and potential solutions. METHODS: Patients and their families who were performing home therapy and who attended the camp of the Korea Hemophilia Association in 2014 were asked to complete questionnaires. The questionnaires pertained to complications of infusion and whether (or not) factor infusion was properly performed. Responses were scored on a scale from 1-5, with a high frequency of complications and adequate performance of infusion being allocated relatively higher scores. RESULTS: The mean score of complications arising from infusion was 1.56±0.46. This was relatively low and was not correlated with the factor infusion training method. The performance of home therapy obtained a relatively high score: 4.46±0.56. The performance score was significantly higher for patients who had practiced infusion with medical personnel, an injection simulator or a video clip. CONCLUSION: Although most patients properly performed home therapy, further improvement is needed in training of infusion and keeping records of bleeds. It is essential to establish guidelines on home therapy, develop a standardized patient and family training program, and reinforce the role of hemophilia treatment centers in educating patients and their families.

Kawasaki Disease with Influenza A Virus and Mycoplasma pneumoniae Infections: A Case Report and Review of Literature

Although an association of Kawasaki disease (KD) with infectious agents has been suggested, none have been proven to cause KD. In this case study, we present a case of KD with concurrent onset of influenza and Mycoplasma pneumoniae (MP) infections. A 27-month-old boy presented with prolonged fever, cough, and rhinorrhea. During the initial testing, influenza A infection was identified, and he was treated with oseltamivir. Despite the antiviral therapy, the fever persisted, and he had cervical lymph node enlargement, bilateral conjunctival injection, fissured red lips, strawberry tongue, and erythematous skin lesions on the Bacillus Calmette-Guérin vaccination site. Thus, the patient was diagnosed with KD and was treated with intravenous immunoglobulin (IVIG). The result of the initial antimycoplasma immunoglobulin M (IgM) antibody testing and was positive, and an increased IgM titer from baseline was found in a repeat test. We reviewed the hypotheses on pathogens known to be associated with KD and the etiology of KD. Based on our findings, we suspect that symptoms of KD and coronary artery lesions can occur from various infections besides those caused by Mycoplasma species and influenza viruses.

A Study on Dietary Habits and Lifestyle of Girls with Precocious Puberty / 대한소아소화기영양학회지

PURPOSE: This study was conducted to analyze the change in the obesity index in girls receiving a gonadotropin-releasing hormone agonist (GnRHa), based on treatment duration, and to aid in nutritional counseling by investigating dietary habits and lifestyle. METHODS: Anthropometric examinations were conducted on 62 girls treated with GnRHa from January 2010 through July 2014. Parents were asked to fill out questionnaires on patient dietary habits and lifestyle. RESULTS: The group taking GnRHa for over 1 year had a higher rate of obesity increase than the group taking GnRHa for less than 1 year, but they had common habits related to obesity, which should be corrected. In addition, 69.2% of the normal weight group taking GnRHa for over 1 year gained weight, and needed more intensive programs, which include physical exercise and nutritional education. Although girls with precocious puberty showed a decrease in the intake of high-calorie foods with nutritional intervention regardless of treatment duration, they still had problems that needed improvement, such as shorter meals and lack of exercise. CONCLUSION: Girls with precocious puberty and their parents should emphasize maintenance of proper body weight, especially when treatment for over 1 year is anticipated. Consistent education in nutrition, ways to increase intensity and duration of physical activity, and the need to slow down mealtimes are important in managing obesity; doctors need to perform regular checkups and provide nutritional counseling.